Surgical management of vitreoretinal complications in X-linked retinoschisis: long-term outcomes in a pediatric population.

PURPOSE: To describe the clinical characteristics, surgical management strategies, and long-term anatomical and functional outcomes of vitreoretinal complications associated with X-linked retinoschisis (XLRS) in a pediatric population.

METHODS

This monocentric retrospective consecutive case series included patients who underwent surgery for XLRS-related vitreoretinal complications. Surgical indications were categorized as retinal detachment (RD), progressive macular schisis, or persistent vitreous hemorrhage (VH).

RESULTS

Thirty-six patients (47 eyes) were included, with a mean age at presentation of 6.8 ± 5.6 years. Surgical indications were RD in 24 eyes (51%), progressive macular schisis in 16 eyes (34%), and VH in 7 eyes (15%). Complete anatomical success for RD was achieved in 75% of eyes after a mean of 2.0 ± 1.0 surgeries, with rhegmatogenous RD showing better outcomes than tractional RD (88% vs. 43%, p = 0.04). Visual acuity improved significantly in eyes with anatomical success. Surgery for progressive macular schisis improved or stabilized retinal structure but did not significantly improve visual acuity. Postoperative complications were frequent, particularly cataract and ocular hypertension, but final retinal attachment was obtained in 44 eyes (94%).

CONCLUSION

Surgical management of XLRS-related vitreoretinal complications results in favorable anatomical outcomes, especially RD, although multiple procedures are often required. Surgery for progressive macular schisis should be reserved for documented progression or fovea-threatening disease. Pars plana vitrectomy has favorable outcomes in non-resolving VH with limited complications.